title: Urinary IgG and α2-macroglobulin are powerful predictors of outcome and responsiveness to steroids and cyclophosphamide in idiopathic focal segmental glomerulosclerosis with nephrotic syndrome.
abstract: OBJECTIVE
  To assess whether high-molecular-weight proteins excretion predicts outcome and therapy-responsiveness in patients with FSGS and nephrotic syndrome.
  
  
  METHODS
  Thirty-eight patients measured at biopsy fractional excretion of IgG (FEIgG) and urinary α2-macroglobulin/creatinine ratio ( α m/C). Low and high risk groups were defined by cutoffs assessed by ROC analysis. In all patients first-line therapy was with steroids alone or in combination with cyclophosphamide.
  
  
  RESULTS
  α2m/C and FEIgG were correlated with segmental sclerosis (r = 0.546; r = 0.522). Twenty-three patients (61%) entered Remission and 9 (24%) progressed to ESRD. Comparing low and high risk groups, by univariate analysis remission was predicted by FEIgG (77% versus 25%, P = 0.016) and α2m/C (81% versus 17%, P = 0.007) and ESRD at best by FEIgG (0% versus 75%, P < 0.0001) and α2m/C (4% versus 67%, P < 0.0001). By multivariate analysis FEIgG was the only independent predictor of remission and α2m/C the most powerful predictor of ESRD. Low and high risk groups of FEIgG and α2m/C in combination had very high predictive value of sustained remission and ESRD in response to therapy.
  
  
  CONCLUSIONS
  FEIgG and α2m/C are powerful predictors of outcome and responsiveness to steroids and cyclophosphamide; their predictive value, if validated in prospective studies, may be useful in clinical practice suggesting first-line alternative treatments in high risk patients.
authors.affiliation: D'Amico Foundation for Renal Diseases Research, Via Cherubini, 6, 20100 Milan, Italy.
authors.forename: Claudio
authors.initials: C
authors.lastname: Bazzi
authors.forename: Virginia
authors.initials: V
authors.lastname: Rizza
authors.forename: Daniela
authors.initials: D
authors.lastname: Casellato
authors.forename: Gilda
authors.initials: G
authors.lastname: Stivali
authors.forename: Gregorio
authors.initials: G
authors.lastname: Rachele
authors.forename: Pietro
authors.initials: P
authors.lastname: Napodano
authors.forename: Maurizio
authors.initials: M
authors.lastname: Gallieni
authors.forename: Giuseppe
authors.initials: G
authors.lastname: D'Amico
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D003520:Cyclophosphamide / Q000008:administration & dosage; D005260:Female; D005923:Glomerulosclerosis, Focal Segmental / Q000188:drug therapy* / Q000473:pathology / Q000652:urine; D006801:Humans; D007074:Immunoglobulin G / Q000652:urine*; D007676:Kidney Failure, Chronic / Q000188:drug therapy / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009404:Nephrotic Syndrome / Q000150:complications / Q000188:drug therapy* / Q000652:urine; D011379:Prognosis; D013256:Steroids / Q000008:administration & dosage; D016896:Treatment Outcome; D000511:alpha-Macroglobulins / Q000652:urine*
chemical_list: D007074:Immunoglobulin G; D013256:Steroids; D000511:alpha-Macroglobulins; D003520:Cyclophosphamide